CASE REPORT

Assessment of choroidal osteoma complicating choroidalneovascularization by optical coherence tomographyangiography

Ceying Shen . Shu Yan . Min Du . Hong Zhao . Ling Shao . Yibo Hu

Received: 8 October 2016 / Accepted: 23 March 2017

� The Author(s) 2017. This article is an open access publication

Abstract

Purpose Choroidal osteoma (CO) frequently leads

to progressive visual loss due to complications of

secondary choroidal neovascularization (CNV).We

report herein the function of optical coherence

tomography angiography (OCTA) in observation of

CO complicating CNV.

Methods A 25-year-old female presented to our

hospital with chief complaint of sudden unilateral

visual acuity decrease for one week, with metamor-

phopsia in the left eye. Her best corrected visual acuity

was 0.12 in the left eye. Then complete ophthalmo-

logical examinations including fundus photography,

B-scan ultrasound, fundus fluorescent angiography,

and spectral-domain optical coherence tomography

(SD-OCT) were performed. She was diagnosed as CO

on the basis of these results. But the diagnosis could not

explain the sudden visual loss and submacular hyper-

reflective lesion by SD-OCT. Furthermore, she under-

went OCTA and indocyanine green angiography.

Results A diagnosis of classic juxtafoveal CNV

secondary to CO was made eventually. Then she was

treated with an intravitreous injection of ranibizumab

twice. The visual acuity got better and better during

the treatment, and the efficacy was stable, giving rise

to both subjective and anatomic improvement.

Conclusions Optical coherence tomography angiog-

raphy has the advantage of varying the segmentation

and scrolling through the different retinal layers, and

layer-specific observation of blood flow in each layer.

In addition, OCTA can measure the vessel area change

of CNV and provide a better appreciation of CNV,

observing the efficacy more elaboratively and quan-

tizedly. OCTA makes promising noninvasive identi-

fication of the CO-related CNV.

Keywords Choroid osteoma � Choroidalneovascularization � Optical coherence tomography

angiography � Fundus fluorescent angiography

Abbreviations

CO Choroidal osteoma

CNV Choroidal neovascularization

C. Shen (&) � S. Yan � M. Du � H. Zhao �L. Shao � Y. HuDepartment of Zhengzhou Second People Hospital,

Ophthalmology, Zhengzhou Eye Hospital, Zhengzhou

Ophthalmic Institution, Zhengzhou Hanghai Middle Road

No. 90, Zhengzhou 450000, China

e-mail: 516045610@qq.com

S. Yan

e-mail: yanshu8302@126.com

M. Du

e-mail: 15890075057@163.com

H. Zhao

e-mail: zhaohong120@hotmail.com

L. Shao

e-mail: shao_95@163.com

Y. Hu

e-mail: yiyi019@126.com

123

Int Ophthalmol

DOI 10.1007/s10792-017-0503-9

OCTA Optical coherence tomography

angiography

FFA Fundus fluorescent angiography

SD-

OCT

Spectral-domain optical coherence

tomography

RAPD Relative afferent pupil defect

ICGA Indocyanine green angiography

RPE Retinal pigment epithelium

Background

Choroidal osteoma (CO) is a benign choroidal tumor

which contains mature bone [1]. It is most present in

young healthy females in the second decade of life [2].

Although most cases are asymptomatic, metamor-

phopsia, blurred vision, and visual field defects may be

initial symptoms [3, 4].The prognosis of the tumor is

deteriorated by the presence of choroidal neovascu-

larization (CNV). In this study, we report herein a case

of CO complicating CNV that was specifically diag-

nosed by optical coherence tomography angiography

(OCTA).

Case presentation

A 25-year-old female presented to our hospital with

chief complaint of sudden unilateral visual acuity

decrease for one week, with metamorphopsia in the

left eye. Her best corrected visual acuity was 1.0 in the

right eye and 0.12 in the left eye. Intraocular pressure

was 14 mmHg in the right eye and 16 mmHg in the

left eye. The grade of relative afferent pupil defect

(RAPD) was 3? in the left eye. There was no other

relevant past ocular, medical or family history. She

underwent complete ophthalmological examinations

including fundus photography, B-scan ultrasound,

fundus fluorescent angiography (FFA), and spectral-

domain optical coherence tomography (SD-OCT).

Fundus photography showed peripapillary subretinal

orange and yellow mass, without involvement of

fovea, but accompanied with submacular blood

(Fig. 1a). B-scan ultrasound delineated a high reflec-

tive echo at the posterior pole with shadowing of the

retrobulbar tissues (Fig. 1b). FFA demonstrated peri-

papillary mottled fluorescence, and macular

hypofluorescence, without late staining indicating

the absence of neovascularization (Fig. 1c). SD-OCT

revealed submacular hyperreflective lesion, bulge of

retinal pigment epithelium (RPE), and nasal choroidal

hyperreflectivity (Fig. 1d). She was diagnosed as CO

on the basis of these results. But the results could not

explain the sudden visual loss and submacular hyper-

reflective lesion. In addition, to further confirm, she

underwent indocyanine green angiography (ICGA)

and OCTA. ICGA showed there was a dark area at the

peripapillary with faint hyperfluorescence at the

macular, indicating the presence of CNV (Fig. 1e).

The instrument used in the OCTA was to obtain

amplitude-decorrelated angiography images (software

version: 2015.1.0.90; Optovue, Inc., Fremont, CA,

USA). OCTA (3 mm 9 3 mm) exhibited that there

was abnormal irregular vascular signal in the outer

retina layer and the choriocapillaris layer, and peri-

papillary hyperreflectivity was present in the chorio-

capillaris layer. The osteoma showed no flow in the

choriocapillaris layer. There was vascular morphology

in the RPE bulge on the B-scan OCTA images

corresponding to the abnormal vascular signal in the

angiography (Fig. 1f). A diagnosis of CO complicat-

ing CNV was confirmed eventually. CNV detected in

the region was responsible for the subretinal fluid. And

the flow area of CNV was 0.024 mm2 visible on

OCTA. Then she was treated with an intravitreous

injection of 0.5 mg ranibizumab (Lucentis;

cFig. 1 Ophthalmological examinations of a 25-year-old female

with choroid osteoma complicating choroidal neovasculariza-

tion. Fundus photography a showed peripapillary subretinal

orange and yellowmass (yellow arrow), without involvement of

fovea, but accompanied with submacular blood. B-scan

ultrasound b delineated a high reflective echo at the posterior

pole with shadowing of the retrobulbar tissues. FFA c demon-

strated peripapillary mottled fluorescence (yellow arrow), and

macular hypofluorescence, without late staining indicating the

absence of neovascularization. SD-OCT d revealed submacular

hyperreflectivity, bulge of RPE (yellow arrowhead), and nasal

choroidal hyperreflectivity (yellow arrow). ICGA e showed

there was a dark area at the peripapillary with faint hyperflu-

orescence at the macular (yellow arrowhead). OCTA images of

3 mm 9 3 mm, f exhibited that there was abnormal irregular

vascular signal in the outer retina layer and the choriocapillaris

layer (yellow arrowhead), and peripapillary hyperreflectivity

was present in the choriocapillaris layer. The osteoma showed

no flow in the choriocapillaris layer. There was vascular

morphology in the RPE bulge on the B-scan OCTA images

corresponding to the abnormal vascular signal in the

angiography

Int Ophthalmol

123

Genentech, South San Francisco, CA). The risks and

benefits of the intravitreal injection of ranibizumab

were explained to the patient, and written consent was

obtained.

Two weeks after intravitreous injection, she came

back to recheck, and the visual acuity got improved.

Her best corrected visual acuity increased to 0.3, with

a reduction of metamorphopsia in the left eye. SD-

Int Ophthalmol

123

OCT revealed that submacular hyperreflectivity

decreased, bulge of RPE disappeared, but nasal

choroidal hyperreflectivity sustained. Abnormal vas-

cular signal was absent in the outer retina layer and the

choriocapillaris layer by using OCTA. This patient

was sensitive to ranibizumab. The surgery yielded

favorable outcomes. One month after operation, her

best corrected visual acuity was 0.3 in the left eye. SD-

OCT revealed the absence of submacular hyperreflec-

tivity and discontinuous ellipsoid zone and interdig-

itation zone, without bulge of RPE. OCTA showed

that abnormal vascular signal was absent in the outer

retina layer and the choriocapillaris layer. She was

then performed the second intravitreous injection of

0.5 mg ranibizumab. One month after the second

injection, the patient complained of an evident reduc-

tion of metamorphopsia and the best corrected visual

acuity increased to 0.4 in the left eye. SD-OCT

revealed that discontinuous ellipsoid zone and

interdigitation zone were in regression, without bulge

of RPE. Abnormal vascular signal was not detected by

OCTA. The visual acuity got better and better during

the treatment, and the efficacy was stable, giving rise

to both subjective and anatomic improvement (Fig. 2).

Discussion

In 1978 Gass et al. first described the disease of CO in

four healthy young women with characteristic oph-

thalmoscopy appearance of a slightly elevated, juxta-

papillary, yellowish orange, choroidal lesion with

well-defined margins [5]. CO frequently causes pro-

gressive visual decline as a result of pigment epithelial

decompensation and atrophy of the fovea, or as a result

of complications of secondary CNV [6]. CNV devel-

ops in 47% of CO patients by 10 years and 56% by

20 years, and it is a major cause of visual impairment

Fig. 2 Optical coherence tomography angiography and SD-

OCT for the patient during the follow-up. Two weeks after

intravitreous injection, abnormal vascular signal was absent in

the outer retina layer and the choriocapillaris layer (yellow

arrowhead) by using OCTA. a SD-OCT, d revealed that

submacular hyperreflectivity decreased, bulge of RPE disap-

peared (yellow arrowhead), but nasal choroidal hyperreflectiv-

ity sustained. One month after operation, OCTA showed that

abnormal vascular signal was absent in the outer retina layer and

the choriocapillaris layer (yellow arrowhead). b SD-OCT,

e revealed the absence of submacular hyperreflectivity, and

discontinuous ellipsoid zone and interdigitation zone, without

bulge of RPE. One month after the second injection, abnormal

vascular signal was not detected (yellow arrowhead) by OCTA.

c SD-OCT, f revealed that discontinuous ellipsoid zone and

interdigitation zone were in regression, without bulge of RPE

(yellow arrowhead)

Int Ophthalmol

123

[2]. When serous retinal detachment is complicated by

CO, it is a recognized association that triggers a search

for possible underlying subretinal neovascularization

[3]. If it was not detected by FFA or OCT, ICGA

should be performed to exclude the subretinal neo-

vascularization. Now we have had a new noninvasive

diagnosis examination to preclude CNV, which is

OCTA.

Optical coherence tomography angiography pro-

vides a high-resolution image of the microvasculature

of the retina at various levels in the retina and choroid

such as superficial capillary plexus, deep capillary

plexus, outer retina, and choriocapillaris in the para-

and peri-foveal areas [7, 8]. The advantage of varying

the segmentation and scrolling through the different

retinal layers, and layer-specific observation of blood

flow in each layer makes OCTA widely used in

clinical. In our patient, the secondary CNV was not

detected during the follow-up. So the change of vessel

area was not observed. We set the layer to the

choriocapillaris level and then operated the moving of

the scan line to detect CNV rigorously. CNV was

detected with OCTA in areas where it was unde-

tectable on the FFA due to the diffuse hyperfluores-

cence. Of note, there have been publications

describing CNV secondary to CO detected by OCTA

[9, 10]. In our study, the CNV showed regression of

lesion size after treatment, same as Szelog et al.

reported [9]. Otherwise, we measured the vessel area

change of CNV, observing the efficacy more elabo-

ratively and quantizedly. Above all, the detection of

CNV secondary to CO is facilitated with OCTA, as the

neovascularization could be covered by osteoma in

dye-based angiography.

The common choroidal tumors misdiagnosed as

CO include choroidal hemangiomas, choroidal mela-

noma, and choroidal metastases. Choroidal haeman-

gioma is a benign vascular tumor, orange in color and

classically located in the macular or paramacular

region of the eye [11]. Associated clinical features are

exudative retinal detachment, macular edema, pig-

mentary changes within the RPE, subretinal fibrosis

over the surface of the lesion, and orange pigment

[12]. Ultrasonography helps to differentiate choroidal

hemangiomas from other choroidal tumors [12].

Choroidal melanoma is suggestive of melanoma such

as greater tumor thickness, the presence of subretinal

fluid and overlying orange pigment, tumor margin

near the optic disk, and the absence of drusen and halo

[13]. OCT generally shows gentle domed shaped,

smooth surface topography with relatively fresh

subretinal fluid demonstrating shaggy photoreceptors

[14]. Choroidal metastases appear as a yellow mass

deep to the retina, often with overlying subretinal fluid

[15].

Conclusions

In the present study, we demonstrate OCTA findings

in patients with CO complicating CNV. It is necessary

to perform noninvasive examinations during the

follow-up to observe the apostasis, replacing the use

of FFA or ICGA. Meanwhile, OCTA can detect the

area change of CNV and provide a better appreciation

of CNV. OCTAmay serve as a marker for patients at a

risk for CNV secondary to CO. And it makes

promising noninvasive identification of the CO-re-

lated CNV. Further study with more number of

patients is still warranted.

Availability of data and materials All the data supporting

our findings are contained within the manuscript.

Compliance with ethical standards

Conflict of interest The authors declare that there is no con-

flict of interest regarding the publication of this paper.

Ethics approval and consent to participate The study par-

ticipant gave oral informed consent for OCTA imaging and

signed informed consent for FFA/ICGA imaging. The study was

approved by the Institutional Review Board of Zhengzhou

Second People Hospital and carried out in accordance with the

tenets of the Declaration of Helsinki.

Open Access This article is distributed under the terms of the

Creative Commons Attribution 4.0 International License (http://

creativecommons.org/licenses/by/4.0/), which permits unre-

stricted use, distribution, and reproduction in any medium,

provided you give appropriate credit to the original

author(s) and the source, provide a link to the Creative Com-

mons license, and indicate if changes were made.

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